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Finally, the mice described here may be especially valuable for testing preventive strategies.
In the Sdhd KO mice described here we saw no development of paraganglioma/pheochromocytoma at any age in two independent cohorts of mice followed for their entire lifespan.
However, the Pink1−/− mice described here show a significant decline in DA levels at a much earlier age than previously reported [17].
Our results suggest that the OBF1 protein level may also be modulated in early B cells, at least in the case of the transgenic mice described here.
Similarly, αB−/− knockout mice did not develop lens opacities or elevated stress-induced light scattering, in dramatic contrast to the lens opacities observed as early as 3 weeks in the αB-R120G knock-in mice described here.
These models are limited by their xenogeneic nature, whereas the DR4 mice described here provide cases of spontaneous disease with morphological and phenotypic similarities to human T cell/histiocyte rich DLBCL and HL with characteristic H/RS-like cells.
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The E50K optineurin transgenic mouse described here exhibited clinical features of POAG and may be useful for mechanistic dissection of POAG and therapeutic development.
In conclusion, while the Sdhd+/− mouse described here is not a model for paraganglioma or pheochromocytoma, the aim to produce such a model remains valid, and should be further explored.
Thus the transgenic mouse described here represents the first example of an animal that could be useful to validate the use of ZF ATF technology for human gene therapy.
The Nav1.8-GIRK2 transgenic mouse described here, or humanisation of mouse GIRK genes in vivo would be a first step in addressing such issues.
In this regard, the chromosome-engineered mouse described here is a model mouse for autism that parallels both phenotypic and genotypic aspects of the human disease.
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